Renal tubular dysfunction in α-thalassemia
Renal tubular dysfunction in α-thalassemia
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DOI：
10.1007/s00467-003-1067-7
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发表时间：
2003-03-01
影响因子：
3
通讯作者：
Vongjirad, A
中科院分区：
文献类型：
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作者：
Sumboonnanonda, A;Malasit, P;Vongjirad, A
红细胞寿命缩短和过量的铁导致地中海贫血患者各种器官系统的功能和生理异常。在早期的研究中，我们发现β-地中海贫血患者肾小管异常的患病率很高。严重程度与贫血程度相关，在接受过量输血和铁螯合治疗的患者中最轻，这表明损伤可能是由贫血和过量铁沉积引起的氧化增加引起的。本研究旨在确定与α-地中海贫血相关的肾脏异常，并将肾脏结果与临床参数相关联。研究了34例患有Hb H疾病或Hb H/Hb CS的儿童患者（平均年龄8.2 ± 2.8岁）。10名患者（第1组）进行了脾切除术，脾切除术后平均持续时间为3.5+/-1.4年; 24名患者（第2组）的脾脏完整。并与15名正常儿童进行比较。与正常儿童相比，两组儿童的尿N-乙酰-β-D-氨基葡萄糖苷酶、丙二醛（MDA）和β（2）-微球蛋白水平显著升高。第1组60%和第2组29%的患者记录到尿蛋白/肌酐比值升高。2例患者（5.9%），每组各1例，有全身性氨基酸尿症。我们发现α-地中海贫血患者近端肾小管异常。增加氧化应激，可能铁诱导，可能发挥重要作用，因为尿MDA水平显着增加，在两组患者。
Shortened red cell life span and excess iron cause functional and physiological abnormalities in various organ systems in thalassemia patients. In an earlier study, we showed that beta-thalassemia patients have a high prevalence of renal tubular abnormalities. The severity correlated with the degree of, anemia, being least severe in patients on hypertransfusion and iron chelation therapy, suggesting that the damage might be caused by the anemia and increased oxidation induced by excess iron deposits. This study was designed to define the renal abnormalities associated with alpha-thalassemia and to correlate the renal findings with clinical parameters. Thirty-four pediatric patients (mean age 8.2+/-2.8 years) with Hb H disease or Hb H/Hb CS were studied. Ten patients (group 1) were splenectomized, with a mean duration post splenectomy of 3.5+/-1.4 years; 24 patients (group 2) had intact spleens. The results were compared with 15 normal children. Significantly higher levels of urine N-acetyl-beta-D-glycosaminidase, malondialdehyde (MDA), and beta(2)-microglobulin were found in both groups compared with normal children. An elevated urine protein/creatinine ratio was recorded in 60% of group 1 and 29% of group 2. Two patients (5.9%), 1 in each group, had generalized aminoaciduria. We found proximal tubular abnormalities in alpha-thalassemia patients. Increased oxidative stress, possibly iron induced, may play an important role, since urine MDA levels were significantly increased in both groups of patients.